Improvement of neurological function in rats with spinal cord injury after the transplantation of neural stem cells directly differentiated from bone marrow mesenchymal stem cells

Objective To study the effect and mechanism of neurological function recovery in rats with spinal cord injury ( SCI) rats after transplantation of neural stem cells which are directly differentiated from bone marrow mesenchymal stem cells ( BMSC ) ,and to investigate the suitable engraftment time. Methods BMSC at 3rd passage were differentiated into neural stem cells ( NSC) , and immunofluorescence staining was used to

Xuefuzhuyu injection induces neuronal differentiation of rat bone morrow mesenchymal stem cells

The primary effective components of Xuefuzhuyu, including Rhizoma Chqanxiong, Radix Salviae Miltiorrhiae, and Radix Angelicae Sinensis, have been shown to induce the differentiation of bone marrow mesenchymal stem cells （BMMSCs） into neuronal-like cells. However, there is little available evidence on the effects of the Chinese herbal compound Xuefuzhuyu on BMMSC differentiation. The present study investigated the effects of Xuefuzhuyu on differentiation of rat BMMSCs into neuronal-like cells, and evaluated the optimal concentration for inducing differentiation. Inverted microscopy was used to observe BMMSCs induced by Xuefuzhuyu; immunocytochemistry revealed expression of the neuronal marker neuron-specific enolase surface antigen in the majority of BMMSCs following treatment with Xuefuzhuyu at concentrations of 1,3, 5 and 10 g/L. A concentration of 3 g/L resulted in highest neuron-specific enolase expression. These results suggest that BMMSCs can be induced to differentiate into neuronal-like cells with Xuefuzhuyu, and 3 g/L is the optimal inductive concentration for effective differentiation.

Neurogenic nitric oxide facilitates the central nociceptive transmission of migraine attacks

Recent studies have shown that nitric oxide （NO） can induce migraine attacks at three possible sites of action： nitroxidergic nerves, the vascular endothelium, and the central nervous system. Most previous studies have focused on the former two sites of action. Several experiments using exogenic NO donors have suggested that nitroglycerin may induce migraine via central mechanisms. However, few studies have investigated the source of the NO involved in the central mechanisms of migraine. The present study used a cat model of migraine to represent migraine attacks in humans. We performed immunochemical staining of successive frozen sections of the brainstem and upper cervical spinal cord, and then used c-Fos protein expression to label nerve cell activation. We observed the effects of N^ω-nitro-L-arginine methyl ester （L-NAME）, a non-selective nitric oxide synthase （NOS） inhibitor, and 7-nitroindozole （7-NI）, a selective neuronal NOS inhibitor, on c-Fos and nNOS expression, which were induced by electrical stimulation to the dura mater near the superior sagittal sinus. The results demonstrated that c-Fos or nNOS immunoreactive cells was concentrated in the superficial layers （laminae Ⅰ and Ⅱ） of the spinal nucleus of trigeminal nerve. L-NAME and 7-NI pre-treatment significantly decreased c-Fos and neurogenic NOS expression; and there was a significant linear correlation between c-Fos and NOS expression （r = 0.858 2, P 〈 0.01）. These findings suggest that neurogenic NO could facilitate migraine nociceptive transmission to second-order neurons of the trigeminal nerve. However, L-NAME and 7-NI may block the activation of neurons in the spinal nucleus of the trigeminal nerve by inhibiting NO synthesis, and thereby attenuate acute migraine attacks.

Nosocomial infection in patients with ventricular hemorrhage cured by lateral ventricle drainage: An analysis of 41 cases

Objective To analyse infection rate of ventricular hemorrhage after lateral ventricle drainage and evaluate the method and measure of precaution. Methods Patients were divided into first and second groups according to the remaining time of drainage tube and into A and B groups according to drainage in one or two sides. After operation the infection condition of insert tube was observed and blood was cultivated regularly. Results The infection rate in the first group was significantly shorter than that in the second one (P 【 0. 01), while it was no significantly difference between groups A and B (P 】 0. 05). Conclusion To remain drainage tube for one to two weeks is safer than for more than two weeks. The infection rate is higher in group with drainage in two sides than in one. In addition, it is obviously related to the patient’s body condition and immunity. 6 refs,2 tabs.

Stroke following off-pump coronary artery bypass in patients with prior stroke

Objective To analyze the risk factors and clinical features of stroke following off-pump coronary artery bypass grafting in patients with prior stroke. Methods From January 2006 to July 2009,the clinical information of 437 patients undergoing OPCABG in Anzhen Hospital

半球卒中患者采用脑对称指数进行持续定量EEG监测

Background and Purpose - There is increased awareness that continuous brain monitoring might benefit neurological patients, because it may allow detection of derangement of brain function in a possible reversible state, allowing early intervention. Here, we explore if quantitative continuous electroencephalography (cEEG) monitoring is technically feasible and possibly clinically relevant in patients with acute ischemic hemispheric stroke. Materials - Twenty- one consecutive patients with an acute hemispheric stroke were monitored in our stroke unit, using cEEG for 12 to 24 hours on the day of admission. EEGs were quantified using a particular measure for symmetry, the brain symmetry index (BSI). This measure was subsequently correlated with the clinical condition of the patient using the National Institute of Health Stroke Scale (NIHSS)- . Results - cEEG was technically feasible. We found a most satisfying positive correlation between the BSI and the NIHSS, with p≈ 0.86 (P < 0.01). Conclusions - Technically, cEEG monitoring posed no major problems. It was found that the BSI correlates satisfactorily with the clinical neurological condition of our stroke patients. This suggests that the BSI can be used as a measure to monitor possible changes of brain function in this patient category.

Ia型遗传性运动感觉神经病的临床严重程度和轴突功能障碍

Background: Hereditary motor and sensory neuropathy type Ia (HMSN Ia) is known as a primarily demyelinating peripheral nerve disease. Evidence is accumulating that axonal involvement determines the course of the disease process. Methods: Fifty- one patients were investigated. Physical disability and impairments were scored. Nerve conduction velocities (NCVs) were used as indirect measures for myelination status and compound muscle/sensory nerve action potential (CMAP/SNAP) amplitudes served as indirect measures for axonal function. Results: Median age was 39 years (range 6- 69). Muscle weakness and sensory dysfunction was more severe in the legs than in the arms and distally more than proximally. However, more than 40 % of the patients had proximal muscle weakness in the legs. Three point grip was used as representative of combined distal arm muscle groups. CMAP amplitude was the most important independent variable in a multiple linear regression model (forward selection) to explain the relation between three point grip strength and four different features, i. e., CMAP amplitude of the abductor pollicis brevis, median nerve MNCV, gender, and duration of signs and symptoms. The severity of axonal dysfunction was nerve length- dependent and was related to the myelination status. The mild physical disability due to both muscle weakness and sensory dysfunction was also related to axonal dysfunction. Conclusions: In HMSN Ia, clinical disease severity at the impairment and disability levels is related to the severity of axonal dysfunction. Our data support the hypothesis that the myelination status is one of the factors that determine the extent of axonal dysfunction later in life. Proximal weakness of the legs is encountered in a considerable proportion of our patients.

帕金森病、绝经期提前与线粒体DNA聚合酶γ突变:临床及分子遗传学研究

Background Mutations in the gene encoding mitochondrial DNA polymerase γ.(POL G), the enzyme that synthesises mitochondrial DNA (mtDNA), have been associated with a mitochondrial disease autosomal dominant or recessive progressive extern al ophthalmoplegia and multiple deletions of mtDNA. Mitochondrial dysfunction is also suspected to participate in the pathogenesis of Parkinsons disease. Howe ver, no primary gene defects affecting mitochondrial proteins causing mendelian transmission of parkinsonism have been characterised. We aimed to analyse the ge ne sequence of POLG in patients with progressive external ophthalmoplegia and th eir healthy relatives. Methods In seven families of various ethnic origins we as sessed patients with progressive external ophthalmoplegia and unaffected individ uals by clinical, biochemical, morphological, and molecular genetic characterisa tion and positron emission tomography (PET). Findings We recorded mutations in P OLG in members of all seven families. Clinical assessment showed significant cos egregation of parkinsonism with POLG mutations (p < 0 0001), and PET findings w ere consistent with dopaminergic neuron loss. Post mortem examination in two in dividuals showed loss of pigmented neurons and pigment phagocytosis in substanti a nigra without Lewy bodies. Furthermore, most women with progressive external o phthalmoplegia had early menopause before age 35 years. The POLG gene defect r esulted in secondary accumulation of mtDNA deletions in patientstissues. Inter pretation Dysfunction of mitochondrial POLG causes a severe progressive multisys tem disorder including parkinsonism and premature menopause, which are not typic

肘部尺神经病变的短节段神经传导研究

The aim of the study was to assess the diagnostic value of short-segment nerve conduction studies (NCS) at 2 cm intervals from 4 cm abov e to 4 cm below the medial epicondyle in a large group of patients with ulnar ne uropathy at the elbow(UNE). Furthermore, we wanted to compare electrodiagnostic and clinical findings. We evaluated 73 arms in 70 patients with UNE and observed the following abnormalities on short segment NCS: focal conduction block (CB) i n 1, focal CB within creased latency change in 34, and increased latency change alone in 25. Short-segment NCS had an additional localizing value in 28 arms of the 37 patients (76%)with motor conduction velocity (MCV) slowing across the e lbow only or with nonlocalizing electrodiagnostic findings. The lesion was locat ed above the elbow in 32 arms (53%), at the epicondyle in 16 arms(27%), and be low the epicondyle in 12 (20%) of the 60 arms with focal CB or increased latenc y change on short-segment NCS. Patients with CB on routine and short-segment N CS had muscle weakness significantly more often than patients without CB. Thus, short-segment NCS are useful in localizing the lesion in patients with UNE and CB on routine NCS and have additional diagnostic value in patients with MCV slow ing across the elbow or with nonlocalizing signs on routine nerve conduction stu dies. We recommend its use in all patients in whom UNE is suspected.

硬脑膜移植相关克-雅病等朊蛋白病的周围神经受累

Objective: To investigate abnormal prion protein (PrP) deposition in the perip heral nervous system (PNS) in human prion diseases. Methods: Eight patients with prion diseases were examined:three with sporadic Creutzfeld-Ja- kob disease (sCJD),two with dural graft associated CJD (dCJD), one with Gerstm ann-Strussler-Scheinker disease (GSS) with a PrP P102L mutation (GSS102), an d two with a P105L mutation (GSS105).An atypical case of sCJD with PrP plaques i n the brain presented clinically with peripheral neuropathy, and showed demyelin ation in 12%of the teased fibres of the sural nerve. The PNS was investigated b y immunohistochemical and western blotting analyses of PrP. Results: In immunohi stochemical studies,granular PrP deposits were detected in some neurones of dors al root ganglia and a few fibres of peripheral nerves and spinal posterior roots in one sCJD and two dCJD patients, but not in GSS102 or GSS105 patients. The at ypical case of sCJD with peripheral neuropathy showed no obvious PrP deposition in the nerves.Western blotting analysis of the PNS from the dCJD patients reveal ed a small amount of protease K resistant PrPin the dorsal root ganglia and peri pheral nerves. Conclusions:Abnormal PrP deposition occurs in the dorsal root gan glia and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are not correlated with clinical manifestation of peripheral neuropathy in CJD.

重症肌无力的治疗与预后:1999～2000年对470例日本患者的回顾性多中心研究

To clarify the current status of treatments and outcomes of patients with myas thenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mea n age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999 -2000. Thymectomy was performed in 319 (68%) of the patients. Patients who rec eived thymectomy were younger (p=0.01) and had more severe disabilities (p < 0.0 1) than patients without thymectomy. Irrespective of receiving thymectomy, most of the patients were administered corticosteroids (64%), other immunosuppressiv e agents (10%), or cholinesterase inhibitors (86%). Of 395 patients followed u p for more than 12 months after treatment (mean 8.0 years), 30%(34%of thymecto mized and 21%of non thymectomized patients) were in remission (no symptoms wit h/without medication), 34%had only ocular symptoms, and the remaining 35%still had weakness of bulbar or limb muscles at the end of follow up. The prognosis of MG in Japan was generally favorable, but despite the frequent use of thymecto my and immunosuppressive treatments, approximately one third of patients still had generalized weakness. More effective or intensive treatments are required to improve the prognosis.

SPTLC2 gene mutation leads to childhood amyotrophic lateral sclerosis:a case report and literature review

Objective To report the clinical characteristics of a case of childhood amyotrophic lateral sclerosis (ALS)caused by SPTLC2 c.778G> A (p.Glu260Lys)mutation.Methods Whole exon sequencing or whole genome sequencing data from 1 936 patients in the ALS cohort of Peking Union Medical College Hospital were screened for SPTLC2 gene mutations.Clinical data.

PLA2G6-related parkinsonism:clinical and genetic characteristics of 6 cases and literature review

Objective To elucidate the clinical and genetic characteristics of PLA2G6-related parkinsonism.Methods The clinical,imaging and genetic data of 6patients with PLA2G6-related parkinsonism admitted to Peking Union Medical College Hospital from January 2015 to December 2022 were retrospectively collected and analyzed.

Clinical,muscle pathology and molecular genetic analysis of myofibrillar myopathy 3 associated with MYOT gene mutation

Objective To analyze the clinical phenotypic characteristics,muscle pathology,genetic mutations and related proteins of myofibrillar myopathy 3 caused by mutation in MYOT gene,and to conduct a literature review and summary of this disease.Methods A retrospective analysis of the clinical phenotypic characteristics.

Analysis of clinical,pathological and genetic features of 3 patients with limb girdle muscular dystrophy type 2A caused by non-canonical splice site mutations in the CAPN3 gene

Objective To investigate the clinical,pathological and genetic features of 3 cases of limb-girdle muscular dystrophy 2A (LGMD2A) caused by non-canonical splice site mutations in the CAPN3 gene.Methods For the 3 LGMD2A patients admitted to Qilu Hospital of Shandong University from July 2016 to July 2018 were selected as the subjects.Clinical data were collected.

The relationship between T_2 white-matter hyperintensities and hemorrhagic transformation and neurological outcome after intravenous thrombolysis with recombinant tissue plasminogen activator in acute ischemic stroke

Objective To investigate the relationship between white-matter hyperintensities (WMHs) and hemorrhagictransformation (HT) and neurological outcome at 3months after recombinant tissue plasminogen activator (rtPA) treatment in patients with acute ischemic stroke.

Effect of modified Dihuang Yinzi recipe and Huatan Tongluo decoction on neurological function of MCAO rats

Objective To observe the effect of Modified Dihuang Yinzi Recipe(MDYR)and Hua-tan Tongluo Decoction(HTD)on neurological function of middle cerebral artery occlusion(MCAO)model rats.Methods Forty SD rats were randomly divided into 5 groups,i.e.,the sham-op-

Factors associated with early neurological deterioration after intravenous thrombolysis with alteplase in patients with acute ischemic stroke

Objective To investigate the risk factors of early neurological deterioration(END)after intravenous thrombolysis(IVT)in patients with acute ischemic stroke(AIS).Methods We screened consecutive AIS patients from January 2006 to May 2015 in Tangshan Gongren Hospital.In this study,all patients were treated with in-

The efficacy and safety of intravenous alteplase and urokinase in elderly stroke patients:a secondary analysis of INTRECIS study

Objective To analyse the efficacy and safety of elderly patients with acute ischemic stroke who underwent intravenous thrombolysis with alteplase(recombinant tissue plasminogen activator,r-tPA)and urokinase(UK)within 4.5 h of onset.Methods Based on the intravenous thrombolysis registry for Chinese ischemic stroke within 4.5 h of onset(INTRECIS)cohort.